Endocrine Abstracts

JOINT3388

Introduction: Malignant metastatic pheochromocytoma is a rare neuroendocrine tumor that originates from chromaffin cells in the adrenal medulla, distant metastases confirm its malignancy. We present a case of a malignant metastatic pheochromocytoma, emphasizing its uncommon evolution.

Case presentation: A 45 year old patient with history of congenital ictyosis presented with decreased visual acuity and headaches dating to one month. Ophthalmic examination showed stage 3 papillary edema, a cerebral MRI showed an expansive intrasellar process bulging into the sphenoid sinus encompassing the cavernous carotid artery. A TAP CT showed left latero-aortic retroperitoneal tissue mass measuring 12.5*12 cm at the expense of the adrenal gland. The diagnosis of pheochromocytoma was mentioned in the face of blood pressure peaks with hypertensive emergency and the Menard triad and confirmed with Urinary methoxyl derivatives>20 times the upper limit of normal and Plasma methoxyl derivatives>10 times the upper limit of normal. MIBG scintigraphy showed: left adrenal pheochromocytoma with bone metastases and at the level of the sella turcica. Our patient was operated two years after diagnosis, Left adrenalectomy with left nephrectomy were performed while bone metastases were left untreated, the operative consequences were simple. The anatomopathological examination showed Morphological appearance and immunological profile compatible with pheochromocytoma. The postoperative evolution was marked by the normalization of blood pressure figures objectified by a blood pressure Holter, no menard triad and normalization of plasma metanephrine (Normetanephrine =1.57 (<1.07nmol/l), Metanephrine <0.1nmol/l (<0.33)). Seven years after diagnosis our patient presents with a conserved general state.

Conclusion: Five year survival rates after surgery for malignant pheochromocytoma vary between 34 and 60% with high risk of recurrence. The particularity of our patient is conserved general state, normalization of metanephrines and stable metastases up to 5 years after surgery. This case highlights the diagnostic and therapeutic complexity and severe systemic implications of malignant pheochromocytoma. While surgical resection of metastases is an option in oligometastatic cases, it was not feasible for this patient. Novel therapeutic strategies, including internal radiotherapy (I131-MIBG), hepatic chemoembolization, systemic chemotherapy, and targeted therapies such as anti-angiogenics (Sunitinib), offer promising alternatives for managing metastatic disease.